RESUMO
Las malrotaciones por bandas de Ladd son un subtipo de anormalidades de la embriogénesis consistentes en prolongaciones fibrosas, producto de una fijación anómala del mesenterio. Se extienden desde el ciego mal rotado hacia el retroperitoneo, pudiendo producir compresión extrínseca del duodeno. En el 90% de los casos la presentación clínica tiene lugar dentro del primer año de vida como un cuadro agudo, en forma de oclusión duodenal o vólvulo de intestino delgado con la consecuente isquemia de este o hernia interna. En la edad adulta, las formas de presentación son menos específicas. Los métodos de referencia ("gold standard") utilizados para el diagnóstico son la seriada gastroduodenal y la tomografía computarizada. El tratamiento quirúrgico consiste en la cirugía de Ladd, cuyo abordaje convencional fue descripto en 1936 por William Ladd. Presentamos el caso de un paciente adulto con un cuadro oclusivo, causado por dicha anomalía, diagnosticado de forma oportuna y resuelto de manera segura por vía laparoscópica. (AU)
Ladd's band malrotations are a subtype of abnormalities of embryogenesis consisting of fibrous extensions, product of abnormal fixation of the mesentery, that goes from the poorly rotated cecum towards the retroperitoneum, which can cause extrinsic compression of the duodenum. In 90% of cases, the clinical presentation takes place within the first year of life, as an acute condition, like duodenal occlusion or small bowel volvulus with its consequent ischemia or internal hernia. In adulthood, the forms of presentation are less specific. The gold standard methods used for diagnosis are gastroduodenal series and computed tomography. Surgical treatment consists of Ladd's surgery, whose conventional approach was described in 1936 by William Ladd. We present ta case of an adult patient with an occlusive presentation, given by this anomaly, diagnosed in a timely manner and safely resolved by laparoscopic approach. (AU)
Assuntos
Humanos , Masculino , Adulto , Adulto Jovem , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Volvo Intestinal/cirurgia , Anormalidades do Sistema Digestório/cirurgia , Obstrução Intestinal/cirurgia , Vômito , Laparoscopia/métodos , Volvo Intestinal/diagnóstico por imagem , Anormalidades do Sistema Digestório/diagnóstico por imagem , Obstrução Intestinal/diagnóstico por imagemAssuntos
Anormalidades do Sistema Digestório , Siringomielia , Canal Anal/anormalidades , Canal Anal/diagnóstico por imagem , Anormalidades do Sistema Digestório/diagnóstico por imagem , Anormalidades do Sistema Digestório/cirurgia , Humanos , Reto/anormalidades , Sacro/anormalidades , Sacro/diagnóstico por imagemRESUMO
Resumen Objetivo Reportar el caso clínico de un quiste de duplicación gástrica en un paciente adulto. Materiales y Método Paciente de sexo masculino de 70 años con una tumoración quística supramesocólica, con síntomas digestivos inespecíficos. La imagenología solicitada no determinó la etiología ni origen de la misma planteándose múltiples diagnósticos diferenciales. Se realizó una laparotomía exploradora. Resultados La laparotomía confirmó el origen gástrico de la tumoración. Se realizó la resección completa de la misma con resección gástrica en forma de gastrectomía atípica. El estudio anatomopatológico de la pieza confirmó un quiste de duplicación gástrica. Discusión Los quistes de duplicación gástrica son una enfermedad congénita rara, se diagnostican principalmente en la etapa neonatal, siendo raro en el adulto. La mayoría son lesiones únicas, no comunicantes, asintomáticas y de difícil diagnóstico preoperatorio siendo habitual el diagnostico definitivo en el intraoperatorio y con el estudio histológico de la pieza. Conclusión El quiste de duplicación gástrica debe tenerse en cuenta en los diagnósticos diferenciales de toda lesión quística supramesocólica. Son lesiones potencialmente malignas siendo la resección completa la que brinda el diagnóstico y tratamiento definitivo.
Aim To report the clinical case of a gastric duplication cyst in an adult patient. Materials and Method 70 year old male patient with a symptomatic supramesocolic cystic lesion. Preoperative imagenology was not able to determine the exact origin or etiology. Several differential diagnosis were made and finally an exploratory laparotomy was performed. Results The laparotomy determined the gastric origin and a complete resection including gastric wall was done. The anatomopathological study confirmed it was a gastric duplication cyst. Discussion Gastric duplication cysts are a rare congenital disease, diagnosed mainly in the neonatal stage, with rare presentation in the adult stage. Most of these lesions are unique, non-communicating, usually asymptomatic and difficult to diagnose preoperatively, with definitive diagnosis being common in intraoperative and histological study of the piece. Conclusion The gastric duplication cyst must be taken into account in the differential diagnosis of any supramesocolic cystic lesion. They are potentially malignant lesions, and complete resection provides the definitive diagnosis and treatment.
Assuntos
Humanos , Masculino , Idoso , Estômago/anormalidades , Cistos/diagnóstico por imagem , Anormalidades do Sistema Digestório/diagnóstico por imagem , Imageamento por Ressonância Magnética , Ultrassonografia , Cistos/congênitoRESUMO
OBJECTIVE: To determine the key sonographic features for the diagnosis of sirenomelia in the first trimester of pregnancy. METHODS: Cases of sirenomelia from several prenatal diagnosis centers were retrospectively identified and reviewed. The diagnosis was established through the detection of fused lower limbs. Additional sonographic findings were also noted. RESULTS: A total of 12 cases were collected. The most striking sonographic finding was the detection of malformed lower limbs, which appeared to be fused and in an atypical position. Nuchal translucency thickness was mildly increased in three cases (25%). An abdominal cyst, representing the dilated blind-ending bowel, was noted in seven cases (58%). Color flow imaging detected a single umbilical artery in six cases (50%) and the associated intra-abdominal vascular anomalies in three cases (25%). No cases of aneuploidy were detected. The pregnancy was terminated in nine cases (75%) and intrauterine demise occurred in the remaining three cases (25%). CONCLUSIONS: The sonographic detection of abnormal lower limbs or an intra-abdominal cyst located laterally during the first-trimester scan may be warning signs of sirenomelia. This should prompt a detailed examination of the fetal lower body and intra-abdominal anatomy, including the main abdominal vessels, in order to look for additional confirmatory findings.
Assuntos
Anormalidades do Sistema Digestório/diagnóstico por imagem , Ectromelia/diagnóstico por imagem , Deformidades Congênitas das Extremidades Inferiores/diagnóstico por imagem , Artéria Umbilical Única/diagnóstico por imagem , Malformações Vasculares/diagnóstico por imagem , Aborto Induzido , Adulto , Feminino , Morte Fetal , Humanos , Intestinos/anormalidades , Intestinos/diagnóstico por imagem , Medição da Translucência Nucal , Gravidez , Primeiro Trimestre da Gravidez , Ultrassonografia Doppler em Cores , Ultrassonografia Pré-NatalRESUMO
Intestinal malrotation displays classic pattern of the small bowel activity on hepatobiliary scintigraphy that could lead to the diagnosis, but only if an interpreting physician looks for it. Presented are 2 cases, one demonstrates diagnostic findings during the first 60-minute phase, whereas the other reveals the diagnosis only on the later sincalide stimulation phase. The currently recommended structured reporting for hepatobiliary scintigraphy does not include examination of bowel activity pattern, which could result in a missed diagnosis. Including interrogation of the small bowel pattern into the structured reporting checklist would ensure consistent detection of this rare but most consequential diagnosis.
Assuntos
Sistema Biliar/diagnóstico por imagem , Anormalidades do Sistema Digestório/diagnóstico por imagem , Volvo Intestinal/diagnóstico por imagem , Fígado/diagnóstico por imagem , Projetos de Pesquisa/normas , Humanos , Masculino , Pessoa de Meia-Idade , Cintilografia , Padrões de ReferênciaAssuntos
Apendicite/etiologia , Anormalidades do Sistema Digestório/complicações , Volvo Intestinal/complicações , Doença Aguda , Apendicite/diagnóstico por imagem , Apendicite/cirurgia , Anormalidades do Sistema Digestório/diagnóstico por imagem , Anormalidades do Sistema Digestório/cirurgia , Humanos , Volvo Intestinal/diagnóstico por imagem , Volvo Intestinal/cirurgia , Masculino , Pessoa de Meia-Idade , Obesidade Mórbida/complicações , Tomografia Computadorizada por Raios XRESUMO
INTRODUCTION: Craniosynostosis and clavicular hypoplasia, delayed closure of the fontanelle, cranial defects, anal and genitourinary abnormalities, and skin (CDAGS), is an infrequent autosomal recessive entity with only 10 cases reported; no associated gene has been identified so far. CASE REPORT: The proband is a 2-year-old Mexican female with brachycephaly, cleft palate, anal malformation with rectovestibular fistula, and clinodactyly of the third toe overlapping the second. At 4 months of age, she developed a disseminated dermatosis with erythematous scaly nummular plaques, elevated keratotic sharp borders with thin to broad flaking, hematic crusts, and keratotic surface in others. The lesions were slightly pruritic and began at the lower limbs with posterior dissemination to the upper limbs, head, and trunk; palms and soles were unaffected. A skin biopsy showed hyperkeratosis, parakeratosis, acanthosis, and perivascular inflammatory infiltration in the upper reticular dermis among other alterations. She also presented mild bilateral neurosensory hypoacusia and enamel dysplasia. Her karyotype was normal. Treatment with topical hydrating creams partially improved the skin lesions at their center, while the sharply keratotic borders remained, giving a clinical resemblance to porokeratosis. DISCUSSION: The patient suffers from CDAGS syndrome but has normal development, and feet abnormality was described in only one other patient. The treatment with topical hydrating creams improved the skin lesions at their center, while porokeratotic characteristics persisted. CDAGS remains a diagnostic challenge; a comparison with previously reported cases is discussed. The timely detection of the syndrome will allow early treatment that may improve the condition of the patients.